Bottom Line:
LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

Background: Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.

Mentions:
Particular LV segments that were positive for LVNC with a NC/C ratio > 2.3 were in similar locations in patients with DMD with EF > 55%, DMD with EF < 55% or LVNC. In all the subjects, the apical segments were more likely to have ratios > 2.3 with segment 16 being the most common segment to be positive. Segment 16 was positive in 73% of the primary LVNC patients, 40% of DMD subjects with an EF < 55%, and 9.1% of DMD subjects with an EF > 55%. The segments most commonly positive in the LVNC population were apical segments 13, 15 and 16. In the DMD population, it was segments 11,12 and 16. These segments correspond to the segments that have been described to have scar tissue burden with LGE analysis [24] (Figure 2).

Mentions:
Particular LV segments that were positive for LVNC with a NC/C ratio > 2.3 were in similar locations in patients with DMD with EF > 55%, DMD with EF < 55% or LVNC. In all the subjects, the apical segments were more likely to have ratios > 2.3 with segment 16 being the most common segment to be positive. Segment 16 was positive in 73% of the primary LVNC patients, 40% of DMD subjects with an EF < 55%, and 9.1% of DMD subjects with an EF > 55%. The segments most commonly positive in the LVNC population were apical segments 13, 15 and 16. In the DMD population, it was segments 11,12 and 16. These segments correspond to the segments that have been described to have scar tissue burden with LGE analysis [24] (Figure 2).

Bottom Line:
LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

Background: Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.